Presentation
Generalized tonic clonic seizures and delayed milestones.
Patient Data
MRI study shows:
- a complete absence of the corpus callosum
- absent cingulate sulcus with the medial hemispheric sulci seen reaching the third ventricle in a radial fashion
- dilated high riding third ventricle
- parallel configuration of the lateral ventricles
On axial views, the ventricles appear similar to a racing car, and in coronal views, they appear similar to a viking helmet or a moose head.
Case Discussion
Agenesis/dysgenesis of the corpus callosum is an anomaly that may occur in isolation or in association with other CNS or systemic malformations.
It may be complete, partial, or atypical:
- with complete agenesis, the corpus callosum is totally absent
- with partial agenesis (hypoplasia), the anterior portion (posterior genu and anterior body) is formed, but the posterior portion (posterior body and splenium) is not. The rostrum and the anterior/inferior genu are also not formed
It is important to be aware of the features of agenesis of the corpus callosum. First, to distinguish it from hydrocephalus and second to instigate a search for further, more serious, anomalies with which it may be associated.
MRI is currently the imaging modality of choice in infants and children with agenesis/dysgenesis of the corpus callosum, even in patients who have previously undergone CT and US examinations.
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